Liver transplant saves life of a teen with a rarest of rare disorder

Crigler Najjar Syndrome, which affects 1 in a million babies, causes perpetual jaundice as the liver does not produce an enzyme that is necessary to break down bilirubin, a yellowish bi-product of body’s natural processes, to an excretable form

A 13-year-old boy Khalid Muhammad had to spend 12 - 15 hours everyday lying on his bed with blue lights over him. He had a rare condition called Crigler Najjar Syndrome, which affects 1 in a million babies. Babies with this condition are perpetually jaundiced as their liver does not produce an enzyme that is necessary to break down bilirubin, a yellowish bi-product of body’s natural processes, to an excretable form.

“The light emitted during phototherapy helps the bilirubin to break down to the excretable form and does the work of the enzyme. However, as the child grows and the skin becomes thicker, they need to stay under the blue light for longer periods,” said Dr Sibbal.

The normal bilirubin count is 1; Khalid’s count was 30 when he underwent a liver transplant at Indraprastha Apollo hospital to reverse the condition. “The unconjugated bilirubin, one of the four types, can go into the brain and cause permanent damage. It can also lead to deafness,” said Dr Anupam Sibbal, group medical director of Apollo Hospitals.

The only cure for the condition is liver transplant. “Although the liver is healthy, it does not produce the particular enzyme needed to break down bilirubin. So, the only treatment is to replace tue liver with a healthier one,” said Dr Sibbal.

To date, the hospital has transplanted livers in five babies to correct the rare condition. Ayanveer Singh, 3-year-old from Kapurthala, Punjab, underwent a transplant last year at the hospital. “Now, he is active, plays a lot and is like any other baby. But, when he was born, for six months the doctors did not know why he had jaundice. All his tests were normal, he was being given phototherapy,but he did not recover even after six months. That’s when we brought him to Delhi and got a diagnosis,” said Prabhjot Singh, his father.

Childhood jaundice is very common and one in 10 children get it at birth. “But, this should resolve within 15 days with phototherapy. If it doesn’t resolve, the underlying cause has to be determined,” said Dr Sibbal.

Khalids parents brought him to India for his treatment after he started showing signs of brain damage. “He was a brigg student and used to come 4 th in his class. But, his performace started dipping. His soeech also became slurrred. That’s when we knew that just phototherapy will not be enough,” said Khalid Abdullah Baturfi Al Khindi, his father, who donated 30% of his liver for the transplant.

Khalid will now be able to go out and play like all of his friends as he would not be restricted to his bed due to phototherapy for extended periods.